REVIEW ARTICLE POSTERIOR URETHRAL VALVE SUDIP DAS GUPTA1, SHARIF MOHAMMAD WASIM UDDIN1, NADIA RABIN2 1Department of Urology, Sir Salimullah Medical College, Mitford Hospital, 2Department of Microbiology, Sir Salimullah Medical College Bangladesh J. Urol. 2018; 21(1): 35-39 Introduction: as well as renal failure, had all been due to the Posterior urethral valve (PUV) is one of the most common obstruction caused by the PUV.In 1913, Young reported causes of lower urinary tract obstruction in male the first clinical case of PUV, before which all the cases neonates. Although not precisely known, its prevalence had been diagnosed postmortem.(14) In 1919, he is reportedly1/8000 to 1/25000 live births.(1,2) PUV has published a report of 36 cases from the papers of that been observed exclusively in boys,(3) but several reports time, 12 being his own patients and the other 24 from in adults have been published.(4-7) The definitions of various other papers.(15) It was in this paper in which many of the disease manifestations have been changed he presented a classification for the PUV. The numbers in recent years. The primary pathology is a mucosal of case reports or case series of the patients continued membrane in the prostatic urethra, although secondary to grow from the early 20th century, so that by 1949, complications of this membrane result in injuries in the there were 207 published cases of PUV worldwide.(16- kidneys and the urinary bladder, which determine the 18) In the last 10 years, several hypotheses have fate of the children with this primary urethral membrane.(8-11) been proposed regarding bladder function and its PUV was first described in 1515 and subsequently relationship with renal function followingcorrection of the observed at autopsies. In 1802, the first definition for primary obstruction, as well as several urodynamic PUV was written and presented in an article on studies and their relation with renal function and lithotomy.(12) The first report in British journals is found treatment options, all of which has led to major progress in the Lancet, in which Dr Budd reported a PUV in a 16- in the field. Still,after 300 years since the initial diagnosis year old boy who had died of renal failure.(13) He stated of this disease, more than one third of the affected that severe dilation of the kidneys and the urinary tract, children develop renal failure. Types of posrerior urethral valves: Figure. Types of posterior urethral valves Correspondences: Sudip Das Gupta, Department of Urology, Sir Salimullah Medical College, Mitford Hospital, Dhaka 35 Bangladesh J. Urol. 2018; 21(1): 35-39 Posterior Urethral Valve The original classification of the types of posterior urethral differentiation, phenotypic changes in glomerular cells valves proposed by Young in 1919 is still in use today. [14], apopotosis, and increased oxidative stress [15] (A) Type I posterior urethral valves (arrow) are mucosal contribute to decreased nephrogenesis and renal folds extending anteroinferiorly from the caudal aspect dysplasia. A key early event appears to be distention of the verumontanum, often fusing anteriorly at a lower and mechanical stretch of the dilated collecting system, level. They are derived from the plicae colliculi and leading to activation of the apoptotic and inflammatory constitute the vast majority of valves. (B) Type 2 posterior cascades [16]. urethral valves (arrow) are mucosal folds extending The decreased number of total nephrons present at birth anterosuperiorly from the verumontanum toward the leads to hyperfiltration injury, exacerbation of the bladder neck. A rare occurrence, they are probably an underlying inflammatory process, renal fibrosis, and effect rather than a cause of bladder obstruction. (C) ultimately renal failure [17, 18]. The early impact of Type 3 posterior urethral valves (arrow) are disc-like altered differentiation and activation of the apoptotic membranes located below the verumontanum and cascade clearly illustrates that the molecular unrelated to it. They constitute a small percentage of mechanisms and alterations in renal architecture which posterior urethral valves. underlie progressive renal failure are established in utero. Pathophysiology of Posterior Urethral Valves Surgical Care Posterior urethral valve (PUV) is the most common cause Surgical care of the patient with PUV varies according of obstructive uropathy leading to renal failure in male to age, bladder status, and renal status. Antenatal neonates [19]. Although the true incidence of PUVs is surgery has been reported in patients diagnosed with not precisely known, PUV is estimated to occur in 1: PUV with the goal of improving postnatal outcomes. 5000 live births [1]. The normal male urethra is Antenatal hydronephrosis is detectable only after renal anatomically divided into the prostatic and membranous development has occurred and urine production has portions (posterior urethra) and the spongy or anterior started. urethra. The urethral crest is a mucosal ridge that gives a specific form to the posterior urethra, and on either With improvement in antenatal ultrasonography, the hope side of the ridge is the prostatic sinus. The urethral crest was that earlier intervention with vesicoamniotic shunting continues below the verumontanum and coalesces in a would improve postnatal renal function. However, small midline bridge. This membrane, extending laterally identification of those patients who may benefit from and downward, eventually vanishes [20]. early intervention remains elusive. To date, improvement in renal function has been difficult to demonstrate, and The classic form of PUV is found in the prostatic urethra, antenatal intervention remains experimental. below or proximal to the verumontanum. Although the precise embryologic mechanism of PUV remains unknown Urinary drainage [21], four theories have been proposed to explain their Urinary drainage may be accomplished by means of development and include hypertrophy of the urethral postnatal primary valve ablation, vesicostomy, or mucosal folds, persistence and continuation of the cutaneous ureterostomies. urogenital membrane [22], abnormal development of the Wolffian or Mullerian duct [23], and fusion of the Postnatal primary valve ablation verumontanum or the posterior urethral roof epithelium [24]. Ideal treatment involves transurethral incision of the PUV during the first few days of life. Current infant Pathogenesis of Renal Dysfunction resectoscopes are available in 8 French and smaller There is no single genetic mutation or biologic model sizes. The valves can be incised at the 12-, 5-, and 7- that reproduces the phenotype of posterior urethral valves o’clock positions, with either a cold knife or an or congenital bladder outlet obstruction. In early work electrocautery. Some surgeons prefer to leave a catheter with fetal sheep, surgical obstruction caused in place for 2-3 days after the procedure. The timing of hydronephrosis within one week and resulted in the postoperative VCUG varies and ranges from several dysplastic changes at term [10]. Further studies days to several months. confirmed the presence of dysplastic changes and Comparison of the posterior urethral diameter with altered nephrogenesis in kidney’s exposed to outlet anterior urethral diameter can provide an objective obstruction during development [11–13]. Altered cellular measure of valve ablation. In most patients, the posterior Bangladesh J. Urol. 2018; 21(1): 35-39 36 Sudip Das Gupta et al urethra is markedly dilated. Postincision diameter should Potential complications of cutaneous ureterostomies, decrease if the incision is successful. The normal all of which are rare, include the following: posterior-to-anterior urethral ratio is approximately 2.3. • Ureteral devascularization Approximately two thirds of patients have successful valve ablation with one procedure, manifested by a • Inadequate drainage postincision ratio of 3.1 or less. [13] One third of patients • Stomal stenosis require a second incision to achieve this level of posterior urethral reduction. Secondary bladder surgery Secondary bladder surgery takes the form of Because approximately one third of patients will require augmentation cytoplasty or continent appendi- a second valve incision, some authors recommend covesicostomy. routine surveillance cystoscopy 1-2 months after the initial incision to evaluate and treat any residual Augmentation cystoplasty [14] valvular obstruction. Indications for bladder augmentation include In a study by Shirazi et al, factors significantly associated inadequately low bladder storage volumes and high with a higher incidence of obstructive remnant leaflets bladder pressures despite anticholinergic medication after valve ablation for PUV included the following [ 15] : and clean intermittent catheterization. The ileum is most commonly used; however, the large bowel, stomach, • Younger age at the time of surgery and ureter are also used, depending on clinical conditions • Hyperechogenicity of renal parenchyma and surgeon preference. • Presence of vesicoureteral reflux (VUR) Before an augmentation procedure is undertaken, the • Grade 4 or 5 reflux preoperatively implications of bladder augmentation should be carefully Vesicostomy reviewed with parent and family. Augmentation should When urethral size precludes safe valve ablation, a only be offered to patients willing to commit to lifelong communicating channel between the bladder and lower intermittent catheterization. abdominal wall (ie, vesicostomy) can